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Summer FDTN Exam 4 > Vasculitides > Flashcards

Vasculitides Flashcards

1
Q

What are vasculitides

A

Inflammatory processes in the walls of affected blood vessels
systemic or constitutional Sx w/ evidence of organ dysfunction

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2
Q

How do large vessel vasculitides present

A

limb claudication
asymetric BP
absence of pulse, bruits, aortic dilation

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3
Q

How do medium vessel vasculitides present

A 
cutaneous nosules 
ulcers 
digital gangrene 
mononeuritis multiplex 
microaneurysms
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4
Q

How do small vessel vasculitides present

A

Purpura, vesicobullous lesions, urticaria, splinter hemorrhages
Necrotizing granulomas, uveitis, scleritis
Glomerulonephritis
Alveolar hemorrhage

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5
Q

What is polymyalgia rheumatica

A

vasculitis associated with GCA
50+
Women mostly

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6
Q

How does polymyalgia rheumatica present

A

stiffness, aching, pain in muscles of neck, shoulder, pelvic girdle
Difficulty combing hair, putting on coat, rising form chair
Fever, malaise, weight loss

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7
Q

How do you diagnose polymyalgia rheumatica

A

Clinically (pain and stiffness)
anemia
elevated acute phase reactants

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8
Q

How do you treat polymyalgia rheumatica

A

Prednisone low dose (10-20) x 2-4 weeks- will respond in 72 hrs!
Slow taper
monitor for GCA development

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9
Q

These markers tell you it is polymyalgia rheumatica and not another vasculitis

A

NO muscle weakness
Does not cause blindness
Responds to low dose prednisone

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10
Q

What is polyarteritis nodosa

A

Necrotizing inflammation of medium vessels and small arteries 2/2 PMN infiltration, may be associated with Hep B (NOT genetics)

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11
Q

Where does polyarteritis nodosa typically show up

A 
skin 
joints
muscles
peripheral nerves
GI
kidney 
(NOT the lungs!)
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12
Q

S/Sx of polyarteritis nodosa are

A

Extremity arthralgia, myalgia, or neuropathy
Skin ulcers*, distal gangrene
Foot drop (peripheral mononeuritis)
Intestinal angina
Renal insufficiency (but NO glomerulonephritis)

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13
Q

Labs for polyarteritis nodosa will show

A

anemia, leukocytosis, elevated ESR
ANCA- Negative
(make sure you test for HBsAg and HBeAg)

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14
Q

To diagnose polyarteritis nodosa you need

A

Tissue biopsy of an involved organ

Angiogram finding aneurysmal dilations in renal, mesenteric, or hepatic arteries

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15
Q

How do you treat polyarteritis nodosa

A

High dose corticosteroids

Refractory: Cyclophosphamide (prophylaxis against P. jirovecii)

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16
Q

What is the prognosis of polyarteritis nodosa

A

No Tx: poor

Tx: 90% 5 yr survival w/ rare remissions

17
Q

Poor prognostic factors in polyarteritis nodosa are

A 
CKD (Cr >1.6) 
Proteinuria (>1g x day)
GI ischemia 
CNS disease 
Cardiac involvement
18
Q

What is Kawasaki disease

A

mucocutaneous lymph node syndrome affecting mostly boys, < 5 (peak 2 y/o)
Likely infectious origin
Will show fever, skin findings, and cardiac abnormalities

19
Q

To diagnose Kawasaki disease, you need __ and 4/5 of these findings

A

Fever (>38.5 x 5 days) PLUS

  • Conjunctivitis (b/l, non-exudative)
  • Rash (early perineal erythema and desquamation; macular morbilliform or targetoid lesions to trunk/extremities)
  • Extremity changes (hand and foot edema)
  • Adenopathy (anterior cervical)
  • Mucositis (strawberry tongue cracked lips)
20
Q

Kawasaki disease lab findings include

A 
Leukocytosis w/ left shift 
Normocytic normochromic anemia 
elevated acute phase reactants 
elevated ALT/AST
Thrombocytosis 
Pyuria 
*Need ECG/Echo to r/o coronary artery aneurysms
21
Q

How do you treat Kawasaki disease

A

2g/kg IVIG admin over 10-12 hrs
80-100mg/kg ASA in 4 divided doses
F/u with ECG

22
Q

What is Granulomatosis Polyangitis (wegener’s granulomatosis)

A

Vasculitis of small arteries and capillaries affecting mostly Upper/Lower respiratory (necrotizing granulomatous lesions), and Kidney (glomerulonephritis)
40-50 y/o
M=W
If not treated, can be fatal

23
Q

S/Sx of GPA are

A 
Nasal congestion 
Sinusitis 
otitis media
mastoiditis
gingivitis
stridor 
Migratory oligoarthritis
Scleritis/anterior uveitis 
Purpura/Dysesthesia
24
Q

Systemic findings in GPA are

A

Cough
Dyspnea
Hemoptysis
Fever, malaise, weight loss

25
Q

On PE of GPA you may find

A 
congestion 
crusting
ulceration
bleeding
perforation of nasal septum 
UA: proteinuria, RBC cells and casts
26
Q

What imaging is needed to diagnose GPA

A

CT! Much better than CXR

27
Q

GPA labs will show

A

ANCA antibodies
Anti-neutrophil cytoplasmic antibodies
Autoimmune vasculitis
Need thoracoscopic lung biopsy to diagnose

28
Q

How do you treat GPA

A 
chemo agent!
Cyclophosphamide + Corticosteroids OR Rituximab + corticosteroids 
PO QD 
Methotrexate
PCP prophylaxis
29
Q

What is eosinophilic GPA (Churg-Strauss syndrome)

A

Vasculitis of small and medium arteries, MC involving the lung
Presents with chronic rhinosinusitis, Asthma*, Eosinophilia (15k+ or >10%), peripheral neuropathy, palpable purpura, subcutaneous nodules, and cardiac Sx (HF, dysrhythmia, pericarditis)

30
Q

Diagnostics for EGPA are

A

ANCA in 30-60%
Consolidations, nodules, or ground glass on CT
GOLD: lung biopsy!

31
Q

How do you treat EGPA

A

Systemic steroids

advanced/refractory: Immunosuppressive (cyclophosphamide)

32
Q

Vasculitis phase complications of EGPA can lead to

A 
cardiac failure, MI
cerebral hemorrhage 
renal failure
GI bleeding
status asthmaticus 
-w/o Tx, 50% mortality w//in 3 months of Sx
33
Q

What is the MC systemic vasculitis in kids

A

IgA vasculitis: Henoch-Schoenlein purpura

IgA deposits around vessel walls

34
Q

Symptoms of Henosh Schoenlein are

A

acute onset fever
palpable purpura to LE and buttocks
Purpura + Arthritis + glomerulonephritis + Abd pain
*(three big highlights: purpura of LE, arthritis of knee/ankle, kidneys hematuria)

35
Q

Labs for Henoch Schoenlein show

A 
elevated IgA 
Direct immunofluorescence (not sure what it shows)
36
Q

How do you treat Henoch Schoenlein

A

Self limited usually, so pt ed, support, analgesics, low dose steroids
We are mainly worried about the HTN

37
Q

What is Behcet’s syndrome

A

Vasculitis affecting A&V of all sizes
Highest in mediterranean, middle eastern, and east asians (silk road disease)
20-40 y/o
Sporadic familial clustering

38
Q

How does Behcet’s present

A

Recurrent, painful oral ulcers (may be urogenital)
Erythema nodosum- like or Folicular rash
Anterior or posterior uveitis

39
Q

How do you treat Behcet’s disease

A

Topical/intralesional steroids for aphthae
Prednisone
Other meds you can try (not as pref): colchicine, MTX, Thalidomide, cyclosporine, TNA-a inhibitors

Summer FDTN Exam 4 (14 decks)

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